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Primary pigmented nodular adrenocortical disease: the original 4 cases revisited after 30 years for follow-up, new investigations, and molecular genetic findings.
[cushing syndrome]
The
original
4
patients
with
Cushing
syndrome
who
underwent
bilateral
adrenalectomy
for
primary
pigmented
nodular
adrenocortical
disease
were
followed
up
for
an
average
of
31
years
to
determine
whether
they
or
any
of
their
primary
relatives
had
developed
Carney
complex
or
its
components
.
None
had
.
Three
of
the
patients
were
alive
and
well
;
the
fourth
had
died
of
an
unrelated
condition
.
All
the
adrenal
glands
contained
multiple
small
,
black
or
brown
cortical
nodules
,
up
to
4
mm
in
diameter
.
The
extracapsular
extension
of
the
micronodules
was
limited
to
the
immediate
pericapsular
adipose
tissue
and
was
not
considered
evidence
of
low
-grade
malignancy
.
Immunocytochemically
,
the
nodules
were
positive
for
synaptophysin
,
inhibin-
A
,
and
melan
A
and
negative
for
vimentin
and
CD
5
6
.
Ki-
67
antibody
stained
the
cytoplasm
of
cells
in
the
micronodules
but
not
that
of
the
atrophic
cortical
cells
.
The
4
patients
had
the
PRKAR
1
A
deletion
that
has
been
associated
with
the
isolated
form
of
primary
pigmented
nodular
adrenocortical
disease
.